The Turkish Journal of Pediatrics 2010 , Vol 52 , Num 6
Clinical Features of Chronic Granulomatous Disease: A Series of 26 Patients from a Single Center
rDivision of Immunology, Department of Pediatrics, Hacettepe University Faculty of Medicine, and 2Division of Immunology, Ministry of Health Ankara Dışkapı Children's Training and Research Hospital, Ankara, Turkey Turul-Özgür T, Türkkanı-Asal G, Tezcan İ, Köker MY, Metin A, Yel L, Ersoy F, Sanal Ö. Clinical features of chronic granulomatous disease: a series of 26 patients from a single center. Turk J Pediatr 2010;52: 576¬581.

Chronic granulomatous disease is a genetically determined immunodeficiency disorder affecting phagocytic cells rendering them unable to kill certain bacteria and fungi. The present study is a single-center retrospective study that aimed to document the clinical course of 26 children, with a median age of 2.5 years, from 21 families diagnosed as chronic granulomatous disease from 1989-2008. A median delay of 39 months was observed between the onset of infections and age at diagnosis. Pneumonia was the most common initial manifestation of the disease followed by lymphadenitis, skin abscess and diarrhea. An AR inheritance was predominant in the study group. All patients received antibacterial and antifungal prophylaxis, resulting in a marked decrease in the incidence of infections. Overall mortality was 19.2%. These results showed that all features in our group (clinical, progression and outcome) were similar to the literature except for the predominance of autosomal recessive form. Keywords : chronic granulomatous disease, consanguinity, neutrophil

Copyright © 2016